SPTCL
MCID: TCL023
MIFTS: 50

T-Cell Lymphoma, Subcutaneous Panniculitis-Like (SPTCL)

Categories: Blood diseases, Cancer diseases, Genetic diseases, Immune diseases, Rare diseases, Skin diseases

Aliases & Classifications for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

MalaCards integrated aliases for T-Cell Lymphoma, Subcutaneous Panniculitis-Like:

Name: T-Cell Lymphoma, Subcutaneous Panniculitis-Like 57 72
Subcutaneous Panniculitis-Like T-Cell Lymphoma 20 58 6 17 70 32
Sptcl 57 20 58 72
Subcutaneous Panniculitic T-Cell Lymphoma 58
Lymphoma, T-Cell 44

Characteristics:

Orphanet epidemiological data:

58
subcutaneous panniculitis-like t-cell lymphoma
Inheritance: Not applicable; Age of onset: All ages;

OMIM®:

57 (Updated 20-May-2021)
Inheritance:
autosomal recessive

Miscellaneous:
variable severity
variable age at onset (median 36 years)
immunosuppressive drugs and hematopoietic bone marrow transplant are effective treatments


HPO:

31
t-cell lymphoma, subcutaneous panniculitis-like:
Inheritance autosomal recessive inheritance
Onset and clinical course variable expressivity


Classifications:

Orphanet: 58  
Rare skin diseases
Rare haematological diseases


External Ids:

OMIM® 57 618398
ICD10 32 C86.3
MESH via Orphanet 45 C537503
ICD10 via Orphanet 33 C83.6
UMLS via Orphanet 71 C0522624
Orphanet 58 ORPHA86884
MedGen 41 C0522624
UMLS 70 C0522624

Summaries for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

GARD : 20 Subcutaneous panniculitis-like T- cell lymphoma (SPTCL) is a rare type of lymphoma that mainly affects the skin. Lymphomas are cancers that result from a type of white blood cell that grows out of control. In SPTCL, people develop multiple painless nodules in the fat layer under the skin and can these cause swelling. This is known as panniculitis. The cause of this type of cancer is unknown, but genetic factors may be involved. It can be diagnosed by a skin biopsy of the affected area. Treatment for SPTCL depends on the symptoms but often involves taking a steroid medication or a medication that lessens the immune response. Some people with more aggressive SPTCL may need chemotherapy. SPTCL can occur in children or adults, but is typically diagnosed in the 30s. For unknown reasons, it occurs more often in women than men. SPTCL is a very slow-growing cancer and does not usually spread beyond the subcutaneous fat layer. About 20% of people with SPTCL will also develop another condition called hemophagocytic lymphohistiocytosis (HLH) in which the body makes too many immune cells. These immune cells attack other cells in the body and cause an enlarged spleen and a decreased level of blood cells in the body.

MalaCards based summary : T-Cell Lymphoma, Subcutaneous Panniculitis-Like, also known as subcutaneous panniculitis-like t-cell lymphoma, is related to peripheral t-cell lymphoma and lymphoproliferative syndrome, and has symptoms including fatigue and fever. An important gene associated with T-Cell Lymphoma, Subcutaneous Panniculitis-Like is HAVCR2 (Hepatitis A Virus Cellular Receptor 2), and among its related pathways/superpathways are NF-kappaB Signaling and IL12-mediated signaling events. The drugs Methotrexate and Mechlorethamine have been mentioned in the context of this disorder. Affiliated tissues include t cells, bone marrow and skin, and related phenotypes are panniculitis and erythematous papule

OMIM® : 57 Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an uncommon form of T-cell non-Hodgkin lymphoma in which cytotoxic CD8 (see 186910)+ T cells infiltrate adipose tissue forming subcutaneous nodules. Both children and adults can be affected, with a median age at diagnosis of 36 years and a female gender bias. Most patients have accompanying systemic features such as fever or flank pain. A subset (about 20%) of patients develop hemophagocytic lymphohistiocytosis (HLH), usually associated with CD8+ T cells rimming adipocytes in the bone marrow. An infectious agent is not identified, and the disorder is believed to result from improperly activated inflammation. Immunosuppressive therapy may be helpful; hematopoietic bone marrow transplantation is usually curative (summary by Gayden et al., 2018). For a general discussion of genetic heterogeneity of HLH, see HLH1 (267700). (618398) (Updated 20-May-2021)

UniProtKB/Swiss-Prot : 72 T-cell lymphoma, subcutaneous panniculitis-like: An uncommon form of T-cell non-Hodgkin lymphoma, in which cytotoxic CD8+ T-cells infiltrate subcutaneous adipose tissue, and rimming adipocytes in a lace-like pattern. Affected individuals typically present with multiple subcutaneous nodules, systemic B-cell symptoms, and, in a subset of cases, autoimmune disorders, most commonly systemic lupus erythematosus. A subset of patients develop hemophagocytic lymphohistiocytosis. SPTCL transmission pattern is consistent with autosomal recessive inheritance with incomplete penetrance.

Related Diseases for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Diseases related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 140)
# Related Disease Score Top Affiliating Genes
1 peripheral t-cell lymphoma 30.8 TNFRSF8 TIA1 GZMB ALK
2 lymphoproliferative syndrome 30.5 TNFRSF8 TIA1 ALK
3 alk-positive anaplastic large cell lymphoma 30.5 TNFRSF8 ALK
4 composite lymphoma 30.4 TNFRSF8 TIA1 GZMB
5 pediatric lymphoma 30.3 TNFRSF8 TIA1 ALK
6 panniculitis 30.3 TNFRSF8 TIA1 HAVCR2 GZMB
7 anaplastic large cell lymphoma 30.3 TNFRSF8 TIA1 GZMB ALK
8 b-cell lymphoma 30.0 TNFRSF8 TIA1 NFKB2 ALK
9 lymphoblastic lymphoma 29.9 TRB TNFRSF8 CD7
10 lymphoma, hodgkin, classic 29.8 TNFRSF8 TIA1 GZMB ALK
11 cutaneous t cell lymphoma 29.6 TRB TNFRSF8 TIA1 GZMB
12 mature t-cell and nk-cell lymphoma 29.6 TNFRSF8 TIA1 GZMB CD7 ALK
13 lymphomatoid papulosis 29.5 TNFRSF8 TIA1 GZMB ALK
14 sezary's disease 29.3 TRB TNFRSF8 TIA1 CD7 ALK
15 precursor t-cell acute lymphoblastic leukemia 29.3 TRG TRB CD7
16 lymphoma 29.3 TNFRSF8 TIA1 NFKB2 HAVCR2 GZMB ALK
17 mycosis fungoides 29.3 TRG TNFRSF8 TIA1 GZMB CD7 ALK
18 lymphoma, non-hodgkin, familial 29.2 TNFRSF8 GZMB CD7 ALK
19 hemophagocytic lymphohistiocytosis 10.8
20 lupus erythematosus panniculitis 10.7
21 cellulitis 10.5
22 primary cutaneous t-cell lymphoma 10.5
23 autoimmune disease 10.4
24 exanthem 10.4
25 erythema nodosum 10.4
26 dermatomyositis 10.4
27 disseminated intravascular coagulation 10.4
28 dermatitis 10.4
29 macrophage activation syndrome 10.4
30 primary cutaneous lymphoma 10.4
31 cytophagic histiocytic panniculitis 10.4
32 hypertriglyceridemia, familial 10.3
33 hemophagocytic lymphohistiocytosis, familial, 1 10.3
34 autoimmune lymphoproliferative syndrome 10.3
35 cutaneous lupus erythematosus 10.3
36 pyoderma 10.3
37 pyoderma gangrenosum 10.3
38 vasculitis 10.3
39 splenomegaly 10.3
40 discoid lupus erythematosus 10.3
41 rare tumor 10.3
42 lymphomatoid granulomatosis 10.2 TIA1 GZMB
43 systemic epstein-barr virus-positive t-cell lymphoma of childhood 10.2 TIA1 GZMB
44 lichen sclerosus et atrophicus 10.2 TIA1 GZMB
45 nocardiosis 10.2
46 exophthalmos 10.2
47 post-transplant lymphoproliferative disease 10.2
48 rapidly involuting congenital hemangioma 10.2
49 lymphocytic gastritis 10.2 TIA1 GZMB
50 nasal cavity lymphoma 10.2 TIA1 GZMB

Graphical network of the top 20 diseases related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like:



Diseases related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Symptoms & Phenotypes for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Human phenotypes related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like:

58 31 (show all 19)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 panniculitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0012490
2 erythematous papule 58 31 hallmark (90%) Very frequent (99-80%) HP:0030350
3 fatigue 58 31 frequent (33%) Frequent (79-30%) HP:0012378
4 fever 58 31 frequent (33%) Frequent (79-30%) HP:0001945
5 skin ulcer 58 31 frequent (33%) Frequent (79-30%) HP:0200042
6 weight loss 58 31 frequent (33%) Frequent (79-30%) HP:0001824
7 hepatosplenomegaly 58 31 frequent (33%) Frequent (79-30%) HP:0001433
8 hemophagocytosis 58 31 very rare (1%) Frequent (79-30%) HP:0012156
9 chills 58 31 frequent (33%) Frequent (79-30%) HP:0025143
10 abnormality of the coagulation cascade 58 31 frequent (33%) Frequent (79-30%) HP:0003256
11 erythematous plaque 58 31 frequent (33%) Frequent (79-30%) HP:0025474
12 splenomegaly 31 very rare (1%) HP:0001744
13 anemia 31 very rare (1%) HP:0001903
14 autoimmunity 31 very rare (1%) HP:0002960
15 pancytopenia 31 very rare (1%) HP:0001876
16 facial edema 31 very rare (1%) HP:0000282
17 hypertriglyceridemia 31 HP:0002155
18 hypofibrinogenemia 31 HP:0011900
19 increased circulating ferritin concentration 31 HP:0003281

Symptoms via clinical synopsis from OMIM®:

57 (Updated 20-May-2021)
Metabolic Features:
fever

Hematology:
anemia (in some patients)
pancytopenia (in some patients)

Abdomen Spleen:
splenomegaly (in some patients)

Neoplasia:
t-cell non-hodgkin lymphoma, subcutaneous

Skin Nails Hair Skin:
panniculitis
erythematous plaques
hemophagocytosis (in some patients)
subcutaneous nodules, diffuse
atypical cytotoxic cd8+ t cells rimming adipocytes

Head And Neck Face:
facial swelling (in some patients)

Immunology:
autoimmunity (in some patients)
increased levels of inflammatory cytokines, including tnf-alpha, il2, and il1b
hemophagocytic lymphohistiocytosis (hlh) (in some patients)
atypical cytotoxic cd8+ t cells rimming adipocytes in bone marrow (in some patients)

Laboratory Abnormalities:
increased triglycerides (in patients with hlh)
hypofibrinogenemia (in patients with hlh)
hyperferritinemia (in patients with hlh)

Clinical features from OMIM®:

618398 (Updated 20-May-2021)

UMLS symptoms related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like:


fatigue; fever

Drugs & Therapeutics for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Drugs for T-Cell Lymphoma, Subcutaneous Panniculitis-Like (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 326)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Methotrexate Approved Phase 4 1959-05-2, 59-05-2 126941
2
Mechlorethamine Approved, Investigational Phase 4 51-75-2 4033
3
Etoposide Approved Phase 4 33419-42-0 36462
4
Dexamethasone Approved, Investigational, Vet_approved Phase 4 50-02-2 5743
5
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 4 1177-87-3
6
Gemcitabine Approved Phase 4 95058-81-4 60750
7
Ifosfamide Approved Phase 4 3778-73-2 3690
8
Cisplatin Approved Phase 4 15663-27-1 84093 441203 2767
9
Mesna Approved, Investigational Phase 4 3375-50-6 598
10
Pegaspargase Approved, Investigational Phase 4 130167-69-0
11
Denileukin diftitox Approved, Investigational Phase 4 173146-27-5
12
Infliximab Approved Phase 4 170277-31-3
13
Methoxsalen Approved Phase 4 298-81-7 4114
14
Tyrosine Approved, Investigational, Nutraceutical Phase 4 60-18-4 6057
15
Aspartic acid Approved, Nutraceutical Phase 4 56-84-8 5960
16
Pirarubicin Investigational Phase 4 72496-41-4
17 Protective Agents Phase 4
18 Immunosuppressive Agents Phase 4
19 Antimetabolites Phase 4
20 Anti-Infective Agents Phase 4
21 Alkylating Agents Phase 4
22 Etoposide phosphate Phase 4
23 Antiviral Agents Phase 4
24 Gastrointestinal Agents Phase 4
25 Hormones Phase 4
26 Antiemetics Phase 4
27 Antineoplastic Agents, Hormonal Phase 4
28 Hormone Antagonists Phase 4
29 glucocorticoids Phase 4
30 Anti-Inflammatory Agents Phase 4
31 BB 1101 Phase 4
32
Isophosphamide mustard Phase 4 100427
33
protease inhibitors Phase 4
34 HIV Protease Inhibitors Phase 4
35
asparaginase Phase 4
36 Clorazepate Dipotassium Phase 4
37 N-Methylaspartate Phase 4
38 Angiogenesis Inhibitors Phase 4
39 Antibodies, Blocking Phase 4
40 Dermatologic Agents Phase 4
41 Photosensitizing Agents Phase 4
42 Interferon alpha-2 Phase 4
43 Interferon-alpha Phase 4
44
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
45
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
46
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
47
Prednisolone acetate Approved, Vet_approved Phase 3 52-21-1
48
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
49
Cytarabine Approved, Investigational Phase 3 147-94-4 6253
50
Daunorubicin Approved Phase 3 20830-81-3 30323

Interventional clinical trials:

(show top 50) (show all 630)
# Name Status NCT ID Phase Drugs
1 A Randomized Controlled Clinical Trial of Apatinib Combined With CHOP Regimen in the Treatment of Newly Diagnosed Peripheral T-cell Lymphoma Unknown status NCT03631862 Phase 4 Apatinib;CHOP Regimen
2 Comparison of Methotrexate Versus Interferon-alfa 2b on Efficacy, Safety and Quality of Life in Patients With Primary Cutaneous T-cell Lymphomas Unknown status NCT02323659 Phase 4 Methotrexate;Interferon Alfa-2b
3 A Multi-Center, Open-Labelled, Pralatrexate Study in Asian Patients With Peripheral T-cell Lymphoma After Prior Therapy Unknown status NCT03150602 Phase 4 Pralatrexate
4 An Open-label,Multicenter Randomised Study of CTOP/ITE/MTX Compared With CHOP as the First-line Therapy for the New Diagnosed Young Patients With T Cell Non-hodgkin Lymphoma Unknown status NCT01746992 Phase 4 Cyclophosphamide 750mg/m2;Vincristine 1.4mg/m2;Doxorubicin 50mg/m2;prednisone 60mg/m2;ifosfamide 2000mg/m2;pirarubicin 50mg/m2;pirarubicin 25mg/m2;Etoposide phosphate 100mg/m2;methotrexate 1500mg/m2
5 A Randomized Controlled Multi-center Clinical Trial on Treatment of Stage Ⅲ/Ⅳ NK/T Cell Lymphoma With DDGP Regiment (Gemcitabine,Pegaspargase,Cisplatin,Dexamethasone) Unknown status NCT01501149 Phase 4 DDGP(cisplatin,dexamethasone,gemcitabine,pegaspargase);Modified SMILE (MTX,DEX,IFO,L-ASP,Etoposide,Mesna)
6 A Randomized Controlled Multi-center Clinical Trial on Treatment of Stage I/II NK/T Cell Lymphoma With DDGP Regiment (Gemcitabine,Pegaspargase,Cisplatin,Dexamethasone) Unknown status NCT01501136 Phase 4
7 Phase IV Randomized Study of Two Dose Levels of Targretin® Capsules in Subjects With Refractory Cutaneous T-Cell Lymphoma Completed NCT01007448 Phase 4 Bexarotene
8 A Randomized Controlled Multi-center Clinical Trial on Treatment of Peripheral T-cell Lymphoma With DGPT Regiment (Gemcitabine,Cisplatin,Prednisone ,Thalidomide ) Completed NCT01664975 Phase 4 GDPT regimen;CHOP regimen
9 Estimation of Carbohydrate Metabolism Disorder Frequency in Hypertriglyceridemia Induced by Bexarotene Treatment of Cutaneous T Cell Lymphoma - Pilote Study Completed NCT01569724 Phase 4
10 A Multicenter Phase III Randomized Double-Blind Placebo-Controlled Study to Evaluate the Efficacy of Two Dose Levels of DAB389IL-2 (9 and 18 Mcg/kg/Day) in Cutaneous T-Cell Lymphoma (CTCL) Patients With Stage Ia-III Disease Who, Following Less Than or Equal to 3 Previous Therapies, Have Recurrent or Persistent Disease That Has Been Biopsy-Documented to Express CD25 Completed NCT00050999 Phase 4 ONTAK
11 UVADEX Sterile Solution in Conjunction With the UVAR XTS Photopheresis System as an Interventional Therapy for the Treatment Of CTCL (Mycosis Fungoides) in Patients With TMN Classification Stage 1A, 1B, 2A Completed NCT00221039 Phase 4 Methoxsalen+ECP
12 Open Label Study of ONTAK® (Denileukin Diftitox, DAB389IL-2) to Estimate Response in Cutaneous T-Cell Lymphoma (CTCL) According to CD25 Status Completed NCT00211198 Phase 4 ONTAK (denileukin difitox, DAB389IL-2)
13 A Multicenter Open-Label Study to Evaluate the Safety and Efficacy of DAB389IL-2 in Cutaneous T-Cell Lymphoma (CTCL) Patients Following Protocol 93-04-10, Protocol 93-04-11, or Protocol 92-04-01 or Who Meet the Requirements for Protocol 93-04-11 Except Have Biopsy-Documented CTCL That Does Not Express CD25 Completed NCT00051012 Phase 4 ONTAK
14 A Research Study to Bank Samples for Future Evaluation to Identify Biomarkers That Predispose Crohn's Disease and Ulcerative Colitis Patients to Develop Hepatosplenic T-Cell Lymphoma (HSTCL) Completed NCT01804166 Phase 4 Infliximab;Golimumab
15 Single-arm, Multi-center Clinical Study of PD-1 Antibody, Chidamide, Lenalidomide and Gemcitabine for Peripheral T-cell Lymphoma Recruiting NCT04040491 Phase 4 PD-1 blocking antibody, chidamide, lenalidomide and gemcitabine
16 Combination Chemotherapy Including Cisplatin, Ifosfamide, Gemcitabine, L-asparaginase, Etoposide and Dexamethasone (PIGLETS Regimen) as Treatment of Newly Diagnosed and Relapsed/Refractory Peripheral T Cell Lymphomas (PTCLs) Recruiting NCT03071822 Phase 4 cisplatin;ifosfamide;gemcitabine;L-asparaginase;etoposide;dexamethasone
17 Single-arm, Multi-center Clinical Study of PD-1 Antibody, Chidamide, Lenalidomide and Etoposide for Relapsed or Refractory Natural Killer/T Cell Lymphoma Recruiting NCT04038411 Phase 4 PD-1 Antibody, chidamide, lenalidomide and etoposide
18 A Clinical Trial of Chidamide Combined With Etoposide in Relapsed or Refractory NK/T-cell Lymphoma Recruiting NCT04490590 Phase 4 Chidamide+ Etoposide
19 Prospective, Randomized Multicentic to Compare PUVA+IFN Alpha 2a vs PUVA Alone in Mycosis Fungoides Stages Ia, Ib or IIa. Terminated NCT00630903 Phase 4 PUVA (8MOP + UVA) + IFN;PUVA (8-MOP + UVA)
20 Phase III Study of CHOP-14 Plus or Minus Alemtuzumab in Peripheral T-cell Lymphoma of the Elderly Unknown status NCT00725231 Phase 3 chemotherapy
21 Compared the Efficacy and Safety of CDOP Combined With Chidamide and CDOP in de Novo Peripheral T Cell Lymphoma Patients Unknown status NCT03023358 Phase 3 chidamide;cyclophosphamide;liposomal doxorubicin;vincristine;prednisone
22 Combination Chemotherapy Using Cisplatin, Gemcitabine, Ifosfamide, Etoposide, L-asparaginase and Dexamethasone (SIMPLE) for Newly Diagnosed and Relapsed/Refractory NK/T Cell Malignancies Unknown status NCT03623087 Phase 3 Cisplatin;Gemcitabine;Etoposide (VP-16);Dexamethasone;L-asparaginase
23 A Prospective , Multicenter, RandomizedPhase III Study of Improving the Efficacy of Treatment in High Risk T Cell Lymphoma Patients Unknown status NCT01788137 Phase 3 chemotherapy (CHOP);chemotherapy(c-ATT)
24 Non-Hodgkin's Lymphoma T Cell Protocol Unknown status NCT00003423 Phase 3 asparaginase;cyclophosphamide;cytarabine;daunorubicin hydrochloride;dexamethasone;etoposide;mercaptopurine;methotrexate;prednisolone;thioguanine;vincristine sulfate
25 A Randomized Phase III Study to Evaluate the Efficacy of Chemoimmunotherapy With the Monoclonal Antibody Campath-1H (Alemtuzumab) Given in Combination With 2-weekly CHOP Versus 2-weekly CHOP Alone and Consolidated by Autologous Stem Cell Transplant, in Young Patients With Previously Untreated Systemic Peripheral T-cell Lymphomas Completed NCT00646854 Phase 3 CHOP14 chemotherapy (cyclophosphamide, hydroxydaunorubicin, vincristin, prednison) plus G-CSF, combined with alemtuzumab;CHOP14 chemotherapy (see specification under Arm B) plus G-CSF
26 INSPIRE: An Internet-based RCT for Long-term Survivors of Hematopoietic Stem Cell Transplantation Completed NCT00799461 Phase 3
27 A Randomized, Open-Label, Phase 3 Trial of Brentuximab Vedotin(SGN-35) Versus Physician's Choice (Methotrexate or Bexarotene) in Patients With CD30-Positive Cutaneous T-Cell Lymphoma Completed NCT01578499 Phase 3 Brentuximab Vedotin;Methotrexate;Bexarotene
28 A Phase II Study of Oral LBH589 in Adult Patients With Cutaneous T-Cell Lymphoma Who Are Intolerant to or Have Progressed on or After Prior HDAC Inhibitor Completed NCT00490776 Phase 2, Phase 3 LBH589
29 Multicentric Study About Treatment of High Grade Peripheral T Cell Lymphoma in Adults. LTP Study Comparison Between VIP ABVD Versus CHOP Completed NCT00970385 Phase 3 CHOP21;VIP/ABVD
30 A Phase II Study of Oral LBH589 in Adult Patients With Refractory Cutaneous T-Cell Lymphoma Completed NCT00425555 Phase 2, Phase 3 Panobinostat
31 A Randomized, Double-blind, Placebo-controlled, Phase 3 Study of Brentuximab Vedotin and CHP (A+CHP) Versus CHOP in the Frontline Treatment of Patients With CD30-positive Mature T-cell Lymphomas Completed NCT01777152 Phase 3 brentuximab vedotin;doxorubicin;prednisone;vincristine;cyclophosphamide
32 A Multi-center, Single Arm, Safety and Efficacy Study of Pralatrexate With Vitamin B12 and Folic Acid Supplementation in Subjects With Relapsed or Refractory Peripheral T-cell Lymphoma Completed NCT03349333 Phase 3 pralatrexate
33 A Phase 3, Randomized, Two-Arm, Open-Label, Multicenter, International Trial of Alisertib (MLN8237) or Investigator's Choice (Selected Single Agent) in Patients With Relapsed or Refractory Peripheral T-Cell Lymphoma Completed NCT01482962 Phase 3 Alisertib;Pralatrexate;Gemcitabine;Romidepsin
34 An Open-Label Therapeutic Exploratory Clinical Trial of HuMax-CD4, a Fully Human Monoclonal Anti-CD4 Antibody, in Patients With Refractory or Relapsed Non-Cutaneous CD4+ T-Cell Lymphoma Completed NCT00877656 Phase 2, Phase 3
35 A Double Blind Randomized Vehicle Controlled Crossover Study to Evaluate the Safety and Efficacy of Topical Naloxone Hydrochloride Lotion 0.5% for the Relief of Pruritus in Patients With the MF or SS Forms of Cutaneous T-Cell Lymphoma Recruiting NCT02811783 Phase 3 Naloxone Hydrochloride Lotion, 0.5%;Placebo Lotion
36 A Clinical Study to Demonstrate Safety and Efficacy of E7777 in Persistent or Recurrent Cutaneous T-Cell Lymphoma Recruiting NCT01871727 Phase 3 E7777 9 mcg/kg
37 Open-label Multi-center Randomized Non-inferiority Study to Compare Efficacy and Safety of Pegylated Liposomal Doxorubicin Versus Doxorubicin for Newly Diagnosed Peripheral T-cell Lymphoma Recruiting NCT03952572 Phase 3 Cyclophosphamide;pegylated liposomal doxorubicin;Vincristine;Prednisone;Doxorubicin
38 Randomized Phase 3 Study Evaluation the Efficacy and Safety of Oral Azacitidine(CC-486) Compared to Investigator's Choice Therapy in Patients With Relapsed or Refractory Angioimmunoblastic T Cell Lymphoma Recruiting NCT03703375 Phase 3 Azacitidine;Romidepsin;Gemcitabine
39 PA-Gemox Regimen Followed by Thalidomide Versus AspaMetDex Regimen in NKTCL Patients:a Randomized, Open-label, Phase 3 Study Recruiting NCT02085655 Phase 3 pegaspargase;Gemcitabine;Oxaliplatin;Methotrexate;Dexamethasone;Thalidomide
40 A Multicenter, Phase 3, Randomized Trial of Sequencial Chemoradiotherapy With or Without Toripalimab (PD-1 Antibody) in Newly Diagnosed Early-Stage Extranodal Natural Killer/T Cell Lymphoma, Nasal Type (ENKTL) Recruiting NCT04365036 Phase 3 Toripalimab;Pegaspargase;Gemcitabine;Oxaliplatin
41 Open-Label, Multi-Center, Randomized Study of Anti-CCR4 Monoclonal Antibody KW-0761 (Mogamulizumab) Versus Vorinostat in Subjects With Previously Treated Cutaneous T-Cell Lymphoma Active, not recruiting NCT01728805 Phase 3 Vorinostat
42 A Phase 3 Multicenter, Randomized, Double-Blind, Placebo Controlled Study to Determine the Efficacy of Topical SGX301 (Synthetic Hypericin) and Fluorescent Bulb-Light Irradiation for the Treatment of Cutaneous T-Cell Lymphoma Active, not recruiting NCT02448381 Phase 3 SGX301 (synthetic hypericin);Placebo
43 Phase 3 Multi-center Randomized Study to Compare Efficacy and Safety of Romidepsin CHOP (Ro-CHOP) Versus CHOP in Patients With Previously Untreated Peripheral T-cell Lymphoma Active, not recruiting NCT01796002 Phase 3 Romidepsin + CHOP;CHOP
44 Randomized Phase 3 Study Evaluating the Efficacy and the Safety of Oral Azacitidine (CC-486) Compared to Investigator's Choice Therapy in Patient With Relapsed or Refractory Angioimmunoblastic T Cell Lymphoma Active, not recruiting NCT03593018 Phase 3 Oral azacitidine;Romidepsin;Bendamustine;Gemcitabine
45 The Efficacy and Safety of Etoposide, Dexamethasone, Peg-asparaginase or Plus Methotrexate With Sandwiched Radiotherapy in the Treatment of Stage I to II Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type Active, not recruiting NCT02631239 Phase 3 Methotrexate;Etoposide;Dexamethasone;Pegaspargase
46 Multi-center Randomized Study to Compare Efficacy and Safety of Decitabine Plus CHOP (D-CHOP) Versus CHOP in Patients With Previously Untreated Peripheral T-cell Lymphoma Not yet recruiting NCT03553537 Phase 3 Decitabine;Cyclophosphamide;Doxorubicin;Vincristine;Prednisone
47 A Randomized, Open-label, Active Controlled, Multi-center Phase 3 Clinical Study to Compare the Efficacy and Safety of Mitoxantrone Hydrochloride Liposome Injection With Chidamide in Patients With Relapsed/Refractory PTCL. Not yet recruiting NCT04668690 Phase 3 Mitoxantrone Hydrochloride Liposome Injection;Chidamide
48 A Multi-center, Randomized, Phase 3 Study of Sequential Pralatrexate Versus Observation in Patients With Previously Undiagnosed Peripheral T-cell Lymphoma Who Have Achieved an Objective Response Following Initial Treatment With CHOP-based Chemotherapy Terminated NCT01420679 Phase 3 Pralatrexate Injection
49 Comparison of Gemcitabine, Oxaliplatin and Pegaspargase and Etoposide, Vincristine, Doxorubicin, Cyclophosphamide and Prednisone as First-line Chemotherapy in Patients With NK/T-cell Lymphoma:a Prospective Randomized Phase III Study Terminated NCT02359162 Phase 3 Gemcitabine;Oxaliplatin;Pegaspargase;Etoposide;Vincristine;Doxorubicin;Cyclophosphamide;Prednisone
50 Diffuse Large B Cell And Peripheral T Cell Non-Hodgkin's Lymphomas (NHL) In The Elderly. Influence Of Prolonged Oral Etoposide Added To CHOP Combination Chemotherapy In Patients With Good Physiological Status. An EORTC Randomized Phase II-III Trial Including Geriatric Assessment And Quality Of Life Terminated NCT00060385 Phase 2, Phase 3 CHOP regimen;EPOCH regimen;cyclophosphamide;doxorubicin hydrochloride;etoposide;prednisone;vincristine sulfate

Search NIH Clinical Center for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Cochrane evidence based reviews: lymphoma, t-cell

Genetic Tests for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Anatomical Context for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

MalaCards organs/tissues related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like:

40
T Cells, Bone Marrow, Skin, Bone, Spleen, Liver, Breast

Publications for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Articles related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like:

(show top 50) (show all 658)
# Title Authors PMID Year
1
Germline HAVCR2 mutations altering TIM-3 characterize subcutaneous panniculitis-like T cell lymphomas with hemophagocytic lymphohistiocytic syndrome. 61 57 6 20
30374066 2018
2
Frequent germline mutations of HAVCR2 in sporadic subcutaneous panniculitis-like T-cell lymphoma. 57 6 61
30792187 2019
3
Rare Cutaneous T-Cell Lymphomas. 61 20
30497671 2019
4
Immunohistochemical detection of ZAP-70 in 341 cases of non-Hodgkin and Hodgkin lymphoma. 61 54
15133473 2004
5
Immunohistochemical detection of BCL-3 in lymphoid neoplasms: a survey of 353 cases. 54 61
15105810 2004
6
Treatment of T-cell non-Hodgkin's lymphoma. 54 61
15233906 2004
7
Unraveling subcutaneous panniculitis-like T-cell lymphoma: An association between subcutaneous panniculitis-like T-cell lymphoma, autoimmune lymphoproliferative syndrome, and familial hemophagocytic lymphohistiocytosis. 61
32894575 2021
8
Treatment considerations in case of subcutaneous panniculitis like T cell lymphoma with hemophagocytic syndrome. 61
33404186 2021
9
Subcutaneous panniculitis-like T-cell lymphoma invading central nervous system in long-term clinical remission with lenalidomide: A case report. 61
33748238 2021
10
[Research Progression of Subcutaneous Panniculitis-like T-Cell Lymphoma--Review]. 61
33554838 2021
11
Subcutaneous Panniculitis-like T-Cell Lymphoma Without Erythema and Subcutaneous Tumors. 61
33642912 2021
12
Subcutaneous panniculitis-like T cell lymphoma presented as erythema nodosum: A case report. 61
33219732 2021
13
A Rare Case of Subcutaneous Panniculitis-Like T Cell Lymphoma with Hemophagocytic Lymphohistiocytosis Mimicking Cellulitis. 61
33428607 2021
14
Subcutaneous Panniculitis-like T-Cell Lymphoma With Hemophagocytic Lymphohistiocytosis Syndrome in Children and Its Essential Role of HAVCR2 Gene Mutation Analysis. 61
33003142 2021
15
Complete response to romidepsin as monotherapy in treatment-resistant subcutaneous panniculitis-like T-cell lymphoma. 61
33294555 2020
16
Familial aggregation of subcutaneous panniculitis-like T cell lymphoma: A case report of monozygotic twin brothers. 61
33080699 2020
17
Subcutaneous panniculitis-like T-cell lymphoma. 61
31975700 2020
18
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with probable mesentery involvement with associated hemophagocytic syndrome (HPS) - how to treat it? 61
32924664 2020
19
A Longstanding, Persistent and Recurrent Case of Cryptogenic Panniculitis. 61
33362504 2020
20
Second primary malignancies in subcutaneous panniculitis-like T-cell lymphoma: a national database study. 61
32359183 2020
21
Rare non-Hodgkin lymphoma of childhood and adolescence: A consensus diagnostic and therapeutic approach to pediatric-type follicular lymphoma, marginal zone lymphoma, and nonanaplastic peripheral T-cell lymphoma. 61
32452165 2020
22
Concurrent Subcutaneous Panniculitis-like T-Cell Lymphoma and B-Cell Acute Lymphoblastic Leukemia in 2 Pediatric Patients. 61
32852399 2020
23
TIM-3 deficiency presenting with two clonally unrelated episodes of mesenteric and subcutaneous panniculitis-like T-cell lymphoma and hemophagocytic lymphohistiocytosis. 61
32285995 2020
24
Treatment of Facial Lipoatrophy Secondary to Subcutaneous Panniculitis-Like T-Cell Lymphoma. 61
30829756 2020
25
Efficacy of ruxolitinib in subcutaneous panniculitis-like T-cell lymphoma and hemophagocytic lymphohistiocytosis. 61
32271897 2020
26
Activated-cytotoxic TCRαβ+CD4+ peripheral T-cell lymphoma with hypodermal localization: Case report of a lymphoproliferative disorder probably evolved from the CD4+ cytotoxic T-cell subpopulation. 61
31595546 2020
27
HAVCR2 mutations are associated with severe hemophagocytic syndrome in subcutaneous panniculitis-like T-cell lymphoma. 61
32005988 2020
28
A retrospective review of cutaneous lymphoma in Botswana. 61
31647120 2020
29
A Case of Atypical Lymphocytic Lobular Panniculitis. 61
32257679 2020
30
Pralatrexate for refractory or recurrent subcutaneous panniculitis-like T-cell lymphoma with hemophagocytic syndrome. 61
31319088 2020
31
Subcutaneous Panniculitis-Like T Cell Lymphoma: Approach to Differential Diagnosis on Cytology. 61
31580741 2020
32
18F-FDG PET/CT-Guided Diagnosis and Evaluation of Treatment Response to Cyclosporine A in Maldistributed Subcutaneous Panniculitis-like T-Cell Lymphoma Without Typical Skin Rash. 61
31833924 2020
33
Primary Cutaneous Gamma-Delta T-Cell Lymphoma With Long-Term Indolent Clinical Course Initially Mimicking Lupus Erythematosus Profundus. 61
32140447 2020
34
[Significance of immune checkpoints in lymphoid malignancies]. 61
33162527 2020
35
Subcutaneous Panniculitis-Like T-Cell Lymphoma Presenting with Full Facial Edema and Hemophagocytic Syndrome. 61
33397572 2020
36
C-C chemokine receptor 4 expression in CD8+ cutaneous T-cell lymphomas and lymphoproliferative disorders, and its implications for diagnosis and treatment. 61
31355940 2020
37
Non-mycosis fungoides cutaneous lymphomas in a referral center in Taiwan: A retrospective case series and literature review. 61
31978091 2020
38
T-cell lymphomas in a tertiary care center of Mangalore. 61
32362615 2020
39
Hemophagocytic Lymphohistiocytosis as the Initial Presentation of Subcutaneous Panniculitis-Like T-Cell Lymphoma: A Rare Case Responding to Cyclosporine A and Steroids. 61
33331177 2020
40
[Clinical Comparative Analysis of Lupus Panniculitis and Subcutaneous Panniculitis-like T-cell Lymphoma]. 61
31880127 2019
41
Clinical features of infantile subcutaneous panniculitis-like T-cell lymphoma. 61
31808238 2019
42
Is cyclosporine a good option for the treatment of subcutaneous panniculitis-like T-cell lymphoma associated with hemophagocytic syndrome? 61
31571617 2019
43
High-throughput Sequencing of Subcutaneous Panniculitis-like T-Cell Lymphoma Reveals Candidate Pathogenic Mutations. 61
31702703 2019
44
Not all swellings are lymph nodes! A case of subcutaneous panniculitis-like T-cell lymphoma. 61
31649225 2019
45
Positron emission tomography-computed tomography in subcutaneous panniculitis-like T-cell lymphoma. 61
31933563 2019
46
Epidemiology of primary cutaneous γδ T-cell lymphoma and subcutaneous panniculitis-like T-cell lymphoma in the U.S.A. from 2006 to 2015: a Surveillance, Epidemiology, and End Results-18 analysis. 61
30951189 2019
47
Subcutaneous Panniculitis-Like T-Cell Lymphoma With Granulomas as the Predominant Feature. 61
30908292 2019
48
Hemophagocytic lymphohistiocytosis: A rare, potentially fatal complication in subcutaneous panniculitis like T cell lymphoma. 61
30289114 2019
49
Pralatrexate Induces Long-Term Remission in Relapsed Subcutaneous Panniculitis-Like T-Cell Lymphoma. 61
31737895 2019
50
Subcutaneous panniculitis-like T-cell lymphoma in a 25-year-old male patient with sickle cell disease. 61
30944066 2019

Variations for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

ClinVar genetic disease variations for T-Cell Lymphoma, Subcutaneous Panniculitis-Like:

6
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 HAVCR2 NM_032782.5(HAVCR2):c.245A>G (p.Tyr82Cys) SNV risk factor 626252 rs184868814 GRCh37: 5:156533787-156533787
GRCh38: 5:157106776-157106776
2 HAVCR2 NM_032782.5(HAVCR2):c.291A>G (p.Ile97Met) SNV Likely pathogenic 626253 rs35960726 GRCh37: 5:156533741-156533741
GRCh38: 5:157106730-157106730
3 HAVCR2 NM_032782.5(HAVCR2):c.302C>T (p.Thr101Ile) SNV risk factor 626254 rs147827860 GRCh37: 5:156533730-156533730
GRCh38: 5:157106719-157106719
4 HAVCR2 NM_032782.5(HAVCR2):c.332G>A (p.Arg111Gln) SNV Uncertain significance 1049012 GRCh37: 5:156533700-156533700
GRCh38: 5:157106689-157106689

UniProtKB/Swiss-Prot genetic disease variations for T-Cell Lymphoma, Subcutaneous Panniculitis-Like:

72
# Symbol AA change Variation ID SNP ID
1 HAVCR2 p.Tyr82Cys VAR_082211 rs184868814
2 HAVCR2 p.Ile97Met VAR_082212 rs35960726
3 HAVCR2 p.Thr101Ile VAR_082213 rs147827860

Copy number variations for T-Cell Lymphoma, Subcutaneous Panniculitis-Like from CNVD:

7 (show top 50) (show all 868)
# CNVD ID Chromosome Start End Type Gene Symbol CNVD Disease
1 13770 1 1 34400000 Loss T-cell lymphomas
2 24155 1 176443955 176516990 Amplification FAM163A T-cell lymphomas
3 24159 1 176545610 176578591 Amplification T-cell lymphomas
4 24161 1 176565573 176565968 Amplification TOR1AIP2 T-cell lymphomas
5 24164 1 176583077 176620868 Amplification TOR1AIP1 T-cell lymphomas
6 24170 1 176655781 176814172 Amplification CEP350 T-cell lymphomas
7 24182 1 176855625 176898824 Amplification QSOX1 T-cell lymphomas
8 24187 1 176931099 176975845 Amplification LHX4 T-cell lymphomas
9 25717 1 193520555 193532978 Amplification CFHR1 T-cell lymphomas
10 25718 1 193520567 193660013 Amplification CFHR2 T-cell lymphomas
11 25732 1 193739978 193768023 Amplification F13B T-cell lymphomas
12 25737 1 193785111 193847224 Amplification ASPM T-cell lymphomas
13 25742 1 193859229 193901308 Amplification ZBTB41 T-cell lymphomas
14 25749 1 193969065 194179242 Amplification CRB1 T-cell lymphomas
15 25760 1 194253042 194475981 Amplification DENND1B T-cell lymphomas
16 25775 1 194601217 194608154 Amplification T-cell lymphomas
17 25778 1 194618174 194630930 Amplification LHX9 T-cell lymphomas
18 25796 1 194933334 195020426 Amplification NEK7 T-cell lymphomas
19 25985 1 195224011 195241732 Amplification ATP6V1G3 T-cell lymphomas
20 25999 1 195339882 195457987 Amplification PTPRC T-cell lymphomas
21 26165 1 197500000 241700000 Gain T-cell lymphomas
22 27801 1 213064983 213651371 Amplification ESRRG T-cell lymphomas
23 27839 1 213992229 214192804 Amplification GPATCH2 T-cell lymphomas
24 27849 1 214193090 214428879 Amplification T-cell lymphomas
25 27883 1 214847047 214893312 Amplification RRP15 T-cell lymphomas
26 35102 1 6020646 6094789 Deletion KCNAB2 T-cell lymphomas
27 35200 1 6096119 6174449 Deletion CHD5 T-cell lymphomas
28 35249 1 6180833 6193921 Deletion RPL22 T-cell lymphomas
29 35283 1 6199801 6202638 Deletion RNF207 T-cell lymphomas
30 35297 1 6215521 6230298 Deletion ICMT T-cell lymphomas
31 35311 1 6236858 6239833 Deletion T-cell lymphomas
32 35323 1 6243122 6255301 Deletion GPR153 T-cell lymphomas
33 35343 1 6258599 6388087 Deletion ACOT7 T-cell lymphomas
34 38878 10 105700000 135374737 Loss T-cell lymphomas
35 41380 10 17300000 40300000 Loss T-cell lymphomas
36 41720 10 22674405 22746545 Deletion SPAG6 T-cell lymphomas
37 42050 10 27727122 27743303 Deletion PTCHD3 T-cell lymphomas
38 42820 10 40300000 82000000 Loss T-cell lymphomas
39 47063 10 89502855 89567897 Deletion ATAD1 T-cell lymphomas
40 47157 10 89613175 89716382 Deletion PTEN T-cell lymphomas
41 47181 10 90023601 90332988 Deletion RNLS T-cell lymphomas
42 47203 10 90414196 90428542 Deletion LIPF T-cell lymphomas
43 47225 10 90572282 90601495 Deletion ANKRD22 T-cell lymphomas
44 47245 10 90651413 90673218 Deletion STAMBPL1 T-cell lymphomas
45 47253 10 90740268 90765522 Deletion FAS T-cell lymphomas
46 47265 10 90955674 90957051 Deletion CH25H T-cell lymphomas
47 47270 10 90963312 91001512 Deletion LIPA T-cell lymphomas
48 47272 10 91051783 91058661 Deletion IFIT2 T-cell lymphomas
49 47275 10 91077733 91090288 Deletion IFIT3 T-cell lymphomas
50 47279 10 91127793 91134942 Deletion T-cell lymphomas

Expression for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Search GEO for disease gene expression data for T-Cell Lymphoma, Subcutaneous Panniculitis-Like.

Pathways for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Pathways related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.44 TNFRSF8 NFKB2 HAVCR2 GZMB
2
Show member pathways
11.18 TRB NFKB2 GZMB
3 10.89 TRB NFKB2

GO Terms for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Cellular components related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 immunological synapse GO:0001772 8.62 HAVCR2 GZMB

Sources for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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